Dor Yeshorim’s Hearing Loss Panel : Should You Get Tested?

The week of Parshas Vayechi, Dor Yeshorim launched an advertising campaign to promote its new hearing loss panel. As “coincidence” would have it, the bar mitzva of our son, who was born with significant hearing loss in both ears, took place on Parshas Vayechi two years ago. In his pshetl (speech), the first half of which is available on YouTube under the title “Vayechi Bar Mitzva Speech – Deafness,” he notes, based on Rav Chaim Shmuelevitz’s Sichos Mussar, how Chushim’s deafness worked to his advantage, and he was the person who killed Eisav, because he didn’t get sucked into the back-and-forth legal argument that the brothers engaged in with Eisav and was therefore able to perceive the situation more clearly.

Dor Yeshorim’s advertising campaign, entitled “Community Alert – Hearing Loss in Newborns,” encourages people to test using their comprehensive hearing panel that screens for over 60 different genetic mutations that cause recessive hearing loss. Their admirable goal is to, with Hashem’s help, “prevent most cases of recessive, genetically inherited hearing loss.”

What is Dor Yeshorim? What should I know about hearing loss? Should I get tested?

While Ashkenazi and Sefardi Jews are not necessarily less healthy or more prone to be carriers of genetic diseases in general, there are many recessive diseases for which Ashkenazim and/or Sefardim are carriers. Furthermore, the halachic imperative to marry a fellow Jew increases the likelihood that both spouses will be carriers for the same recessive condition.

Dor Yeshorim is an organization founded in 1983 by Rabbi Yosef Ekstein, who, with his wife, tragically lost four children to Tay-Sachs, a rare genetic disorder carried by approximately one in 30 Ashkenazi Jews. Tay-Sachs is agonizingly painful for the child and parent alike, as affected children often experience paralysis, seizures, difficulty swallowing, vision and hearing loss, loss of brain function, and other symptoms, and generally die by the age of four or five. There is no cure for Tay-Sachs currently.

To reduce the incidence of recessive diseases like Tay-Sachs in the Jewish community, Rabbi Ekstein pioneered a groundbreaking, confidential, premarital testing system that screens for genetic diseases prevalent in the community. Each boy and girl gets an ID number. Each potential shidduch candidate calls with both the boy’s and girl’s ID numbers and, after comparing their testing results, both parties are informed whether they are compatible (without disclosing whether anyone is a carrier for a particular disease).

It is hard to overstate the impact that Dor Yeshorim has had; they have prevented thousands of incompatible matches and have played a leading role in virtually eradicating Tay-Sachs and significantly reducing the prevalence of several other severe and potentially fatal disorders within the Orthodox Jewish community.

While some genetic disorders are fatal or severely debilitating, others are relatively mild. Which should people be tested for? Is this a slippery slope, one that could lead to testing for disorders that are merely inconvenient? Would we test for diabetes or obesity? Where does Dor Yeshorim draw the line? Especially given that many are also concerned about the “shidduch crisis,” an increase in the number of genetic disorders being screened would undoubtedly result in otherwise suitable matches not being redt.

Rabbi Ekstein was quoted in Mishpacha magazine (Rachel Ginsberg, “The Gene Marker’s Promise,” Feb. 9, 2011) as saying: “We will only screen for extremely debilitating disorders like cystic fibrosis or Bloom Syndrome, or fatal diseases like Tay-Sachs and Canavan…. We are not looking for a ‘perfect’ gene pool. We used to test for Gaucher’s, but that has been dropped because it has been found to be often asymptomatic, and there is now a successful treatment protocol…. When we started there was no treatment.”

Hearing loss is not one-size-fits-all but, rather, a spectrum ranging from mild to moderate to severe to profound. People with mild and moderate hearing loss can generally hear most sounds but may have trouble hearing low sounds or catching every word in a conversation, particularly in a noisy environment. Those with severe and profound hearing loss have difficulty hearing in general. Historically, those with mild and moderate hearing loss often wore bulky hearing aids that amplified sound but did not reduce background noise, and they therefore still encountered some difficulty hearing; even with speech therapy, they often did not speak nearly as clearly as those without hearing loss. Before the 1980s, those with significant hearing loss may have also worn hearing aids, but generally communicated primarily by using sign language. People with significant hearing loss often experienced difficulty on multiple levels, including socially and vocationally. Unfortunately, some frum people with hearing loss did not feel that they fit in in the religious Jewish community.

In today’s day and age, hearing loss is no longer the infirmity it used to be. Since the 1980s, Hashem has bestowed upon us amazing, miraculous, technological advances. Cochlear implants and bone-anchored hearing aids, which didn't previously exist, can effectively bypass the affected part of the ear and deliver clear signals to the brain; like today’s hearing aids, they are now sleeker, more discreet, and often covered by insurance. Blue-tooth technology can allow sound from phones, computers, and other devices to be transmitted directly to assistive hearing devices. Speech-to-text software enables spoken language to be read in near real-time.

Improved diagnostics (such as the newborn hearing screenings that Dor Yeshorim notes) allow for hearing loss to be caught earlier (often shortly after birth) and for more timely and targeted interventions (including programmable, assistive hearing devices and speech therapy regimens tailored to the particular children’s needs).

As a result, many children who previously would have experienced significant delays in picking up language, communicated mainly in sign language, and been left out socially can now hear and speak clearly and fit right in at mainstream schools and camps. There are also many promising new technologies and treatments in store for the future, iy”H, some already in development. For example, there are phone and computer apps being developed that would allow the user to him- or herself diagnose hearing issues, adjust settings on hearing devices (for instance, to reduce background noise), and convert foreign languages to text.

Our son has bilateral cochlear implants. With Hashem’s help, his hard work, and the love and attention of his parents, he has not only survived but thrived in mainstream yeshivos since he was a toddler. He was an honors student in middle school and is currently attending a top-notch mesivta high school. I myself am deaf in one ear (something tells me our hearing loss might be genetic…) but would like to think I’ve done okay for myself, too. Our son, like Chushim, is deaf. He, like all of us, has had challenges in life, including having had to go to many speech therapy sessions as a toddler and needing to adapt to his circumstances, but he has not “suffered.”

I started with three questions, but I’ve only answered two of them. I don’t think I should be answering my third question (“Should I get tested?”) for you. It’s generally accepted that even parents cannot dictate to their children who they may or may not marry (see, e.g., Rama, Yoreh Deah 240:25). Whom one marries and, by extension, whether one gets tested are personal decisions. Hopefully, you are now armed with more information and can make a more-informed decision for yourself.